Paper - Cysts of the genital ducts Müllerian and Wolffian (1946)

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Smith E. and Strasberg A. Cysts of the genital ducts, Müllerian and Wolffian. (1946) Can Med Assoc J. 55(2): 119–121. PMID 20323857

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This 1946 paper by Smith and Strasberg describes abnormal development of the kidney and genital systems.



Modern Notes: renal abnormalities | genital abnormalities

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Cysts of the Genital Ducts, Mullerian and Wolffian

By Emerson Smith, M.D. and Alex Strasberg, M.D.

Department of Urology, Royal Victoria Hospital, Montreal

Introduction

It is generally agreed that congenital anomalies are common in the genito-urinary system, yet very little can be found in the literature about cysts of the genital ducts. We have recently encountered three such cases on our service, and because of their unusual interest we are reporting them.

Retrovesical and subtrigonal cysts in the male are classified as Miillerian duct cysts. They are all remnants of the Mullerian duct which opens into the utricle of the prostate, and therefore occur only in the male. One subtrigonal cyst in our series occurred in a female, and from an embryological standpoint must be categorized as a Wolffian duct cyst. Clinically the cysts in the male simulate vesical obstruction; in the female, bladder symptoms and persistent pyuria.


- gression of hydroureter


CASE 1

A.B., male, aged 9 years: admitted April 28, 1941, with the following symptoms. Vomiting, abdominal pain, dysuria, difficulty in voiding, fever and rapid pulse. Physical examination essentially normal. Intravenous pyelography showed marked hydronephrosis and hydroureter, left. Non-functioning right kidney. At cystoscopy there was a large round mass on the left side of the bladder extending down to the vesical neck, causing obstruction and obscuring the left ureteral orifice. Operation: suprapubic cystotomy and excision of subtrigonal cyst. A large cystic mass was found in the region of the trigone, which when incised appeared to consist of several smaller cysts. These extended up under the mucous membrane of the posterior surface of the bladder, and did not involve the ureteral orifices. Cystic areas were completely excised. Pezzer catheter inserted. Pyelo-nephritis occurred during convalescence but yielded readily to chemotherapy. Cystoscopy previous to discharge from hospital showed an inflamed mucosa and some slough on the floor of the bladder. No obstruction present at vesical neck. Left kidney normal.

CASE 2

J.D., male, aged 72 years: this case having been previously reported only a brief synopsis will be given here.

Admitted May 6, 1943. Complaint, acute retention. The history, physical examination and laboratory findings were consistent with those found in an ordinary case of prostatism with retention. The salient features, however, were those demonstrated by the barium enema and fluoroscopy. A persistent mass was seen filling the pelvis and displacing the rectum and sigmoid markedly to the right. The bladder seemed to lie anterior to the pelvis.


Fig. 1. (Case 1).—Preoperative. Intravenous pyelography.


Note marked dilatation of left ureter and Fig. 2. (Case 1). — Postoperative. Marked reand hydronephrosis, left. Fig. 3. (Case 2).—Barium enema. The subtrigonal mass is displacing the rectum to the right. Fig. 4. (Case 2). — Cystogram shows bladder to be lying anteriorly in almost normal position.



mass. May 14, 1943. Suprapubic cystotomy with evacuation of subtrigonal cyst. November 30, 1943, readmitted because of aggravation of symptoms. December 3, 1943, patient was transferred to the service of Dr. Gavin Miller, who performed a radical excision of the cyst. A large cyst was found filling the pelvis and adherent to the rectum and bladder. The cyst was freed from adhesions. The greatest difficulty was encountered in freeing it from the trigone, where it was fixed but not incorporated. It would appear that the whole cyst was removed. On the fourth postoperative day the patient developed bronchopneumonia, from which he succumbed on December 11, 1943. Autopsy was not granted.

CASE 3

C.G., female, aged 3% years: complaints; painful micturition, 2 months; pyuria and dysuria; hematuria, two days 1941-42. At the age of ten months patient had an attack of pyuria and painful micturition with complete recovery. She had a. similar attack with hematuria last winter. General physical examination was essentially negative. Cystoscopy revealed a plumsized elevation on the right side of the trigone displacing the ureteral orifice laterally. Indigo carmine injected intravenously showed no accessory ureteral orifices. Both ureters catheterized. The specimen from the right kidney contained pus ++; left was normal. Retrograde and intravenous pyelograms revealed a normal left kidney, but a hydronephrotie right kidney. Cystogram showed a filling defect to the right side of base of bladder. No other abnormality noted. Operation; excision of cyst of trigone. Midline suprapubic incision, bladder opened at vault. To the right side of trigone was a spherical body with a wide pedicle the size of a blue plum. About 30 c.c. cloudy fluid was aspirated from the cyst. Cyst excised level with floor of bladder. Usual closure of bladder; about F. 30 Pezzer catheter. Convalescence was uneventful and intravenous pyelogram six months later revealed a normal urinary tract.


Fig. 5. (Case 3).—Preoperative. graphy. 3).—Postoperative. Right pelvis practically normal.

Intravenous pyeloMarked hydrcnephrosis, right. Fig. 6. (Case Same patient 6 months later.

Embryology

The genital and urinary systems are very intimately associated in their evolution. They originate from mesoderm. In the early fetus there is present both male and female characteristics, that is Miillerian and Wolffian ducts. In the embryo of the third month the sex is

already established and the ducts of the opposite sex are atrophying.

Fate of the Miillerian ducts: the two ducts meet and fuse to form the uterus and vagina, whereas in the male embryo they disappear. The utricle in the prostate in the adult is the only remnant of their embryonic existence.

Fate of the Wolffian ducts: they form the epididymis, vas deferens and ejaculatory duct. The seminal vesicles, ureters and collecting tubules also arise from the Wolffian duets which open at each side of the prostatic utricle. In the female embryo the Wolffian ducts run towards the body of the uterus and disappear by the third month. Occasionally, however, remnants of the distal parts of the ducts persist. Their openings are sometimes in the vulva or vagina.

Congenital anomalies, arrested development and non-obliteration of the remnants of ducts are inseparable. When the Miillerian duct persists in the male it gives rise to a subtrigonal cyst because of its close relation to the bladder.

Eight cases of Miillerian duct cysts have been reported in the literature, to these we add the two male cases in our series. A review of the literature does not reveal any subtrigonal cysts occurring in the females,

Professor C. P. Martin, Department of Anatomy, McGill University,® in explaining the origin of pelvic cysts, states that ‘‘when the upper part of the urogenital sinus dilates to form the bladder it takes up the Wolffian duct at a short distance from the sinus, and when this taking up process reaches the opening of the ureter, it proceeds to take up the lower end of the ureter also, but does not proceed further along the Wolffian duct itself. Thus it comes about that the openings of the ureters and the Wolffian ducts are separated by a considerable distance. While this taking up process is going on the Wolffian duet makes a considerable loop downwards, and the ventral or ascending limb of this loop is actually in the posterior wall of the urogenital sinus.* Later on, the anterior and ventral wall of this loop disappears and the duct opens into the sinus at a much lower level. But if the ventral wall of the ascending limb persisted over a considerable area, it would give rise to a eyst. This, I think, may indeed have been the origin of many cysts, usually described as eysts of the Miillerian ducts.’’

We believe that the female case in our series is a Wolffian duct cyst, and had its origin as here detailed. Wesson and Voelker speak also of the Wolffian ducts as being the origin of subtrigonal cysts in males.


Fig. 7. — Formation of bladder. The two diagrams show the change of level of the entry of the Wolffian duct. The W.D. originally opens near the ureter, it then folds down in a loop, fusing in front with epithelial wall of the sinus, so that it runs up embedded in this. The second diagram shows (dotted lines) the epithelium giving way leaving the duct opening lower down. But if the ventral wall of the ascending limb persisted over a considerable area, it could give rise to a cyst.


(From Fraser’s Embryology, Fig. 259.)

Coppridge? states that only eight cases of subtrigonal eysts have been reported in the literature. The most recent reported cases of Miillerian duct cysts are those of Coppridge, Hennessy, and Campbell. Coppridge’s case was diagnosed after operative procedure was instituted, which consisted of partial exe!sion and fulguration of the remainder of the sac. nessy? reported a case of symmetrical midline tumour in a man 36 years of age with symptoms of bladder obstruction. Abdominal exploration revealed the mass to be firmly attached to the bladder wall posteriorly. The case reported by Campbell? was traecd to the prostatic utricle. There have been other cases reported as cysts of the seminal vesicle, eysts of the prostate and urethra, which we believe cannot be included as cysts of the Miillerian ducts. All of the cases of subtrigonal cysts reported in the literature have failed to reveal any case of Wolffian duct cyst in a female. We therefore believe that our case is unique. The only reference to Wolffian duct is made by Wesson and Voelker,® 7 who hint at the origin of cystic, prostatic, and vesical cysts from both Miillerian and Wolffian remnants.

Diagnosis

This is based on the symptomatology, cystoscopy, cystogram, intravenous pyelography, and barium enema. The symptoms are usually those of bladder neck obstruction. There is frequency, dysuria, and urgency. The subvesical tumour may oceasionally be large enough to cause urinary retention. In the male the mass is usually symmetrical whereas in the female it may lie more on one or other side of the midline. The Wolffian ducts are lateral to the Miillerian ducts which fuse in the midline. Visualization of the upper urinary tract may reveal dilatation of the ureter and pelvis, either unilateral or bilateral. These cysts should be differentiated from ureterocele, and prostatism with bladder symptoms. Cysts of the seminal vesicle nearly always contain spermatozoa.

Treatment

Radical excision of as much of the cyst as possible. Total removal of the sae will be found difficult because of the incorporation of the cyst in the bladder wall. Suprapubic cystotomy seems to be the easiest approach. Intraperitoneal abdominal operations are equally unsuccessful in the complete eradication of the sac. Some authors resort to fulguration of the remnants of cysts. In the literature reviewed there were no recurrences.

Summary and Conclusions

Three cases of subvesical cysts are reported, two in the male, one in the female.

The male cases are Miillerian duct cysts. The female ease is, from an embryological standpoint, a Wolffian duct cyst.

Pelvic tumours and symptoms of urinary obstruction should make one think of eysts of the genital ducts. They should be differentiated from a ureterocele and prostatism.

The treatment consists of the excision of as much of the eyst as possible.

References

1. CopPRIDGE, W. M.: Miillerian duct cysts, South. M. J., 32: 248, 1939.

2. HENNESSY, R. A.: Miillerian duct cysts, J. Urol., 42: 1042, 1939. |

3. CAMPBELL, M. F.: Pediatric Urology, Vol. 1, Macmillan Co., Toronto, Ontario, 1937.

4. Frazer, J. E.: Manual of Embryology, Bailliére, Tindall & Cox, 1931.

5. MARTIN, C. P.: Personal communication.

6. VoELCKER, F.: Chirurgie der Samenblassen, Neue Deutsche Chir., 2: 195, 1908. .

7. WESSON, M. B.: Cysts of the prostate and urethra, J. Urol., 13: 605, 1925.